Gain of function mutant p53 p53 network p53 reactivation tumorigenesis. But similar to bone marrow transplant from a parent, the immune restoration was incomplete. It results from a mutation in the IL-2 receptor gamma gene ( IL-2RG ). Early results from trials of gene therapy for X-SCID resulted in life-saving correction of T lymphocytes. Not only will this strategy abrogate mutant p53 GOF, but it will also restore WT p53 tumor-suppressive functions. An engineered virus brings a healthy copy of the gene into the stem cells to replace the mutated gene that causes the disease. Thus, we suggest that targeting mutant p53, via its reactivation to the wild-type form, may serve as a promising therapeutic strategy for many cancers that harbor mutant p53. Finally, we discuss mechanisms by which "Mother Nature" tries to abrogate the pro-oncogenic functions of mutant p53. Because of a gene mutation, babies who are born with X-linked severe combined immunodeficiency (X-SCID) do not develop immune cells properly, leaving them highly susceptible to infections. It is one of the most serious primary immunodeficiency disorders with early death due to disturbed or absent T and B cell functions. In the current review, we discuss the various GOF effects of mutant p53, and how it may serve as a central node in a network of genes and proteins, which, altogether, promote the tumorigenic process. Severe combined immunodeficiency disease (SCID) is an inherited primary immunodeficiency disorder that presents by six months of age with opportunistic infections caused by bacteria, viruses, fungi, and protozoa. Over time, they may develop: recurrent or severe. Infants with SCID usually appear healthy at birth. These genes encode three proteins that are components of a multimeric transcriptional complex, RFX, which binds a sequence named an X box, present in the promoter of all MHC. The genes mutated in Groups B, C, and D are named RFXANK, RFX5, and RFXAP. ![]() ![]() A growing body of evidence suggests that these pro-oncogenic functions of mutant p53 proteins are mediated by affecting the transcription of various genes, as well as by protein-protein interactions with transcription factors and other effectors. Severe combined immunodeficiency (SCID) is a group of inherited genetic disorders characterized by a profound deficiency in cellular and humoral immunity arising from one of many T-cell maturation defects in the bone marrow or thymus gland. One of these, named the MHC class II transactivator, or CIITA, is the gene mutated in Group A. Aside from losing the tumor-suppressive functions of the wild-type form, mutant p53 proteins often acquire inherent, novel oncogenic functions, a phenomenon termed mutant p53 gain-of-function (GOF). The p53 protein is mutated in about 50% of human cancers.
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